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    Extragonadal Germ Cell Tumors Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Cellular Classification of Extragonadal Germ Cell Tumors

    Extragonadal germ cell tumors can be benign (teratoma) or malignant. The latter group can be divided into seminoma and nonseminoma germ cell tumors, which include the following:

    • Embryonal carcinomas.
    • Malignant teratomas.
    • Endodermal sinus tumors.
    • Choriocarcinomas.
    • Mixed germ cell tumors.

    Extragonadal germ cell tumors occur much more commonly in males than in females [1] and are usually seen in young adults. They are aggressive neoplasms and can arise virtually anywhere, but typically the site of origin is in the midline (mediastinum, retroperitoneum, or pineal gland). Gonadal origin should be excluded by careful testicular examination and ultrasound. The diagnosis can be difficult and should be considered in any patient with a poorly defined epithelial malignancy, particularly young individuals with midline masses.[2,3]

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    An international germ cell tumor prognostic classification has been developed based on a retrospective analysis of 5,202 patients with metastatic nonseminomatous germ cell tumors and 660 patients with metastatic seminomatous germ cell tumors.[4] All patients received treatment with cisplatin-containing or carboplatin-containing therapy as their first chemotherapy course. The prognostic classification, shown below, was agreed on in early 1997 by all major clinical trial groups worldwide and should be used for the reporting of clinical trials' results of patients with extragonadal germ cell tumors.

    Good Prognosis

    Nonseminoma

    • Testis/retroperitoneal primary

      and

    • No nonpulmonary visceral metastases

      and

    • Good markers - all of:
      • AFP less than 1,000 ng/mL

        and

      • hCG less than 5,000 iu/L (1,000 ng/mL)

        and

      • LDH less than 1.5 x upper limit of normal

    56% of nonseminomas

    5-year progression-free survival (PFS) rate of 89%

    5-year survival rate of 92%

    Seminoma

    • Any primary site

      and

    • No nonpulmonary visceral metastases

      and

    • Normal AFP, any hCG, any LDH

    90% of seminomas

    5-year PFS rate of 82%

    5-year survival rate of 86%

    Intermediate Prognosis

    Nonseminoma

    • Testis/retroperitoneal primary

      and

    • No nonpulmonary visceral metastases

      and

    • Intermediate markers - any of:
      • AFP 1,000 ng/mL or greater and 10,000 ng/mL or less

        or

      • hCG 5,000 iu/L or greater and 50,000 iu/L or less

        or

      • LDH 1.5 × N or greater and 10 × N or less
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